Cardiomyopathy explained to help shine a light on a health condition.

Sometimes it takes a shock event – such as the sudden death of a much-loved celebrity – to shine a light on a health condition.

The news this month that George Michael’s untimely death on Christmas Day, aged just 53, happened as a result of dilated cardiomyopathy with myocarditis (inflammation of the heart muscles) and a fatty liver is a prime example – and, as it turns out, there’s good reason to put it in the spotlight.

Cardiomyopathy is a condition that can affect babies, children and adults of all ages, and sometimes symptoms go unnoticed.

DIFFERENT TYPES

There are a number of different types of cardiomyopathy; the three main ones are hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC).

“HCM is an inherited disease, where the muscle wall of your heart becomes thickened and stiff. This makes it harder for the heart to pump blood out and around your body. Common symptoms include chest pain, palpitations, light headedness and fainting,” explains Emily Reeve, senior cardiac nurse at British Heart Foundation (BHF).

“DCM is a disease of your heart muscle, where it becomes stretched, thin and ‘baggy’. Like HCM, this means the heart’s unable to pump blood around your body efficiently. In most cases, DCM develops slowly, so some people can have quite severe symptoms before they’re diagnosed. The most common symptoms are shortness of breath, swelling of the ankles and abdomen, excessive tiredness and palpitations.

“ARVC affects the cells of the heart muscle that are held together by proteins. In people with ARVC, these proteins do not develop properly and so cannot keep the heart muscle cells together. The muscle cells become detached and fatty deposits build up in an attempt to repair the damage.”

George Michael’s death at 53 was a result of dilated cardiomyopathy.

Reeve explains that HCM affects one in 500 people in the UK, and is the “most common cause of sudden death in young people”, although most people with it have few, if any, symptoms. DCM affects one in every 250 people (20-35% of people inherit the condition), while ARVC, which is more rare, affects one in 2,000.

“Worryingly, the majority are undiagnosed and unaware they may be at risk of a deadly heart attack or cardiac arrest. In fact, 80% of ARVC cases are found in post mortem,” adds Reeve, noting that BHF has warned the overall figure could be much higher due to it being underdiagnosed, along with “undiscovered faulty genes which can increase a person’s risk of these potentially fatal conditions”.

And the risks don’t just apply to adults; in babies and under-16s, “cardiomyopathy is a leading cause of heart muscle failure or heart rhythm disorders”.

“The cause of cardiomyopathy in children often remains unknown, and diagnosis is complicated because of a large number of rare genetic causes and an array of specialised diagnostic tests,” says Reeve. “The signs of the disease can also be variable and symptoms are often not specific to these conditions.”

WHAT CAN YOU DO?

Where genes are involved, cardiomyopathy can run in families. “Someone with a cardiomyopathy like HCM has a 50% chance of passing on this gene to their child,” notes Reeve.

Generally speaking though, taking care of your heart is certainly worthwhile. There are numerous potential causes, including uncontrolled high blood pressure, certain viral infections, other conditions that may affect heart muscles (including Wegener’s granulomatosis and sarcoidosis), poor diet, alcoholism and drug abuse.

“Keeping your heart healthy, whatever your age, is the most important thing you can do to help prevent and manage heart disease that isn’t inherited,” stresses Reeve. “Eat a healthy, balanced diet, don’t smoke and exercise regularly.”

It’s also important to get any potential symptoms properly checked out.

“If any new symptom occurs, make an appointment to speak to your doctor,” says Reeve. “Those with a known family history of cardiomyopathy should be referred to a specialist inherited cardiac conditions service for screening. Genetic testing is a big decision for someone to make, and it’s important they, and other family members, have the right support to guide them through this.

“When getting screened, you’ll be given genetic counselling, as often cardiomyopathy does not present itself until later on in life. Unfortunately, besides having a heart transplant, there is currently no cure for cardiomyopathy, but you may be provided with medication or a pacemaker, to control your symptoms and prevent complications.”

:: Concerned about your heart health, or conditions affecting your family? Call BHF’s Heart Helpline on 0300 330 3311 (Mon-Fri, 9-5pm). Visit www.bhf.org.uk